Quantification of mRNAs encoding proteins of the glycosphingolipid catabolism in mouse models of GM2 gangliosidoses and sphingolipid activator protein precursor (prosaposin) deficiency

Astrid Potratz; Silke Huttler; Uwe Beierfreund; Richard L. Proia; Kunihiko Suzuki; Konrad Sandhoff

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Quantification of mRNAs encoding proteins of the glycosphingolipid catabolism in mouse models of GM2 gangliosidoses and sphingolipid activator protein precursor (prosaposin) deficiency

机译：在GM2神经节苷脂酶和鞘脂激活蛋白前体（prosaposin）缺乏症的小鼠模型中编码糖鞘脂分解代谢蛋白的mRNA的定量

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We have investigated the mRNA amounts of six lysosomal proteins (β-hexosaminidase α- and β-subunit, sphingolipid activator protein precursor, GM2 activator protein, lysosomal sialidase, β-glucocerebrosidase) involved in the degradation of glycosphingolipids. We analyzed extracts from brain tissues of mouse models for lysosomal storage diseases, i.e., the GM2 gangliosidoses and the deficiency of the sphingolipid activator protein precursor (prosaposin). The mRNA levels were quantified by real-time reverse transcription-polymerase chain reaction. Although storage of the respective lysosomal proteins has been reported in human and mice, no increase of their mRNA amounts could be detected here. Our results indicate that there is no transcriptional upregulation of lysosomal proteins in the examined neuronal storage disorders.

机译：我们研究了参与糖鞘脂降解的六种溶酶体蛋白（β-己糖胺酶α-和β-亚基，鞘脂激活蛋白前体，GM2激活蛋白，溶酶唾液酸酶，β-葡萄糖脑苷脂酶）的mRNA量。我们分析了小鼠模型脑组织中的溶酶体贮积病，即GM2神经节苷脂和鞘脂激活蛋白前体（prosaposin）缺乏症的提取物。 mRNA水平通过实时逆转录-聚合酶链反应定量。尽管已经报道了在人和小鼠中各自溶酶体蛋白的储存，但是在这里不能检测到它们的mRNA量的增加。我们的结果表明在所检查的神经元存储障碍中没有溶酶体蛋白的转录上调。

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《Biochimica et biophysica acta. Molecular basis of disease: BBA》 |2000年第3期|共7页
作者
Astrid Potratz; Silke Huttler; Uwe Beierfreund; Richard L. Proia; Kunihiko Suzuki; Konrad Sandhoff;
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正文语种 eng
中图分类生物工程学（生物技术）;
关键词
sphingolipidosis; mRNA quantification; real-time reverse transcription-polymerase chain reaction; lysosomal hydrolase; sphingolipid activator protein; knock-out mouse;

机译：鞘脂异常;mRNA定量;实时逆转录聚合酶链反应;溶酶体水解酶;鞘脂活化蛋白;敲除小鼠;

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1. Quantification of mRNAs encoding proteins of the glycosphingolipid catabolism in mouse models of GM2 gangliosidoses and sphingolipid activator protein precursor (prosaposin) deficiency [J] . Astrid Potratz, Silke Huttler, Uwe Beierfreund, Biochimica et biophysica acta. Molecular basis of disease: BBA . 2000,第3期

机译：在GM2神经节苷脂酶和鞘脂激活蛋白前体（prosaposin）缺乏症的小鼠模型中编码糖鞘脂分解代谢蛋白的mRNA的定量
2. Additional biochemical findings in a patient and fetal sibling with a genetic defect in the sphingolipid activator protein (SAP) precursor, prosaposin. Evidence for a deficiency in SAP-1 and for a normal lysosomal neuraminidase [J] . A Poulos, B C Paton, B Kustermann-Kuhn, The biochemical journal . 1992,第2期

机译：鞘脂激活蛋白（SAP）前体prosaposin中存在遗传缺陷的患者和胎儿同胞的其他生化发现。 SAP-1缺乏和正常的溶酶体神经氨酸酶的证据
3. Lysosomal proteolysis of prosaposin, the precursor of saposins (sphingolipid activator proteins): Its mechanism and inhibition by ganglioside [J] . Hiraiwa M, Martin BM, Kishimoto Y, Archives of Biochemistry and Biophysics . 1997,第1期

机译：prosaposin的溶酶体蛋白水解，saposins的前体（鞘脂激活蛋白）：其机制和神经节苷脂的抑制作用
4. Identification of iron-deficiency-inducible genes encoding transcription-related proteins in graminaceous plants [C] . R. N. Itai, H. Inoue, Y. Ogo, International Plant Nutrition Colloquium . 2005

机译：禾本科植物中编码转录相关蛋白的铁缺乏诱导基因的鉴定
5. The beta amyloid protein precursor of Alzheimer's disease: Analysis of mRNAs and protein products. [D] . Palmert, Mark Raney. 1990

机译：阿尔茨海默氏病的β淀粉样蛋白前体：mRNA和蛋白产物的分析。
6. Additional biochemical findings in a patient and fetal sibling with a genetic defect in the sphingolipid activator protein (SAP) precursor prosaposin. Evidence for a deficiency in SAP-1 and for a normal lysosomal neuraminidase. [O] . B C Paton, B Schmid, B Kustermann-Kuhn, 1992

机译：鞘脂激活蛋白（SAP）前体prosaposin具有遗传缺陷的患者和胎儿同胞的其他生化发现。 SAP-1缺乏和正常的溶酶体神经氨酸酶的证据。
7. Quantification of mRNAs encoding proteins of the glycosphingolipid catabolism in mouse models of GM2 gangliosidoses and sphingolipid activator protein precursor (prosaposin) deficiency [O] . Potratz Astrid, Hüttler Silke, Bierfreund Uwe, 2000

机译：在GM2神经节苷脂酶和鞘脂激活蛋白前体（prosaposin）缺乏症的小鼠模型中编码糖鞘脂分解代谢蛋白的mRNA的定量

Quantification of mRNAs encoding proteins of the glycosphingolipid catabolism in mouse models of GM2 gangliosidoses and sphingolipid activator protein precursor (prosaposin) deficiency

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